Extrahepatic portal vein obstruction in children of the first year of life: diagnostic and treatment approaches

Abstract

Extrahepatic portal vein obstruction (EHPVO) is an important cause of symptomatic portal hypertension (PH) in pediatric population. PH may lead to serious complications including variceal bleeding and hypersplenism. Literature data on infants under 12 months old with EHPVO is poor. Aim - to analyze our experience in treatment of children with symptomatic PH in children of the first year of life, the peculiarities of the course of EHPVO in them, and acknowledge readers with diagnostic algorithm of patients with EHPVO that ensures the timely start of treatment and reduce the risk of life-threatening complications. Materials and methods. 11 patients who were diagnosed with EHPVO before age of 12 month are included into the study. All patients underwent in-hospital treatment in National Specialized Children’s hospital “Okhmatdyt” in a period since 2006 to 2022. Results. Among 11 patients of the group, 7 (63.6%) were males. Mean age at diagnosis was 6.02±0.67 months. 9 (83.8%) patients had umbilical catheter in anamnesis. 7 (63.6%) patients manifested bleeding episodes. Ultrasonography and enhanced computed tomography was performed in all patients. Endoscopically all patients had high grade varices. 8 (72.7%) patients underwent surgical interventions. Median hospital stay duration in children with bleeding was 36 (95% CI: 28-56) days, and 17 (95% CI: 14-22) days in children without bleeding (p<0.003). The success of primary surgical interventions was assessed with Kaplan-Meyer test. Median overall survival was reached at 920 days after initial surgery; primary surgical intervention survival 0.417 (95% CI: 0.205-0.072). There was zero mortality in the study group. Conclusions. Newborns and infants with anamnesis of umbilical catheterization should be followed up closely by ultrasound imaging specialist. Patients with suspected PH are to be immediately referred to endoscopy and computed tomography in specialized center. Timely diagnosis, current endoscopic band ligation success in variceal bleeding primary prophylaxis would ensure further successful surgery and shorter hospital stay. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.